Beta-glucuronidases are members of the glycosidase family of enzymes that catalyze breakdown of complex carbohydrates.Human β-glucuronidase is a type of glucuronidase (a member of glycosidase Family 2) that catalyzes hydrolysis of β-D-glucuronic acid residues from the non-reducing end of mucopolysaccharides (also referred to as glycosaminoglycans) such as heparan sulfate. Human β-glucuronidase is located in the lysosome. n the gut, brush border β-glucuronidase converts conjugated bilirubin to the unconjugated form for reabsorption. Beta-glucuronidase is also present in breast milk, which contributes to neonatal jaundice. The protein is encoded by the GUSB gene in humans and by the uidA gene in bacteria. Deficiencies in β-glucuronidase result in the autosomal recessive inherited metabolic disease known as Sly syndrome or Mucopolysaccharidosis VII. A deficiency in this enzyme results in the build-up of non-hydrolyzed mucopolysaccharides in the patient. This disease can be extremely debilitating for the patient or can result in hydrops fetalis prior to birth.
Background References
1. Dubot P. et. al. First Report of a Patient with MPS Type VII, Due to Novel Mutations in GUSB, Who Underwent Enzyme Replacement and Then Hematopoietic Stem Cell Transplantation. Int J Mol Sci. 2019 Oct.
2. Bonecker S. et. al. Is the BCR-ABL/GUSB transcript level at diagnosis an early predictive marker for chronic myeloid leukemia patients treated with imatinib? Rev Bras Hematol Hemoter. 2015 Mar-Apr.
Sequence Similarity
Belongs to the glycosyl hydrolase 2 family.
Post-translational Modification
N-linked glycosylated with 3 to 4 oligosaccharide chains.
Western blot analysis of Beta glucuronidase on different lysates with Rabbit anti-Beta glucuronidase antibody (ET7110-66) at 1/5,000 dilution.
Lane 1: U-937 (Human acute monocytic leukemia cell) cell lysate Lane 2: THP-1 (Human acute monoblastic leukemia cell) cell lysate Lane 3: K-562 (Human chronic myelogenous leukemia cell) cell lysate Lane 4: MCF7 (Human breast cancer cell) cell lysate
Lysates/proteins at 15 µg/Lane. Exposure time: 62 seconds; ECL: K1801
Blocking: 5% NFDM/TBST, 1 hour at room temperature Primary antibody: ET7110-66, 1/5,000 in primary antibody dilution buffer 5% NFDM/TBST, overnight at 4 ℃ Secondary antibody: Goat anti-Rabbit IgG-HRP (HA1001), 1/50,000 in 5% NFDM/TBST, 1 hour at room temperature
Predicted band size: 74.7 kDa Observed band size: 75 kDa
Immunohistochemical analysis of paraffin-embedded human prostate carcinoma tissue with Rabbit anti-Beta glucuronidase antibody (ET7110-66) at 1/200 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 8.0-8.4) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET7110-66) at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.
Immunohistochemical analysis of paraffin-embedded human kidney tissue with Rabbit anti-Beta glucuronidase antibody (ET7110-66) at 1/200 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 8.0-8.4) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET7110-66) at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.
Flow cytometric analysis of Beta glucuronidase was done on THP-1 cells. The cells were fixed, permeabilized and stained with the primary antibody (ET7110-66, 1/50) (red). After incubation of the primary antibody at room temperature for an hour, the cells were stained with a Alexa Fluor 488-conjugated Goat anti-Rabbit IgG Secondary antibody at 1/1000 dilution for 30 minutes.Unlabelled sample was used as a control (cells without incubation with primary antibody; black).
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"